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Desmoid-Type Fibromatosis: A Front-Line Conservative Approach to Select Patients for Surgical Treatment

Annals of Surgical Oncology volume 16, pages 2587–2593 (2009)Cite this article

Abstract

Purpose

Surgery is still the standard treatment for desmoid-type fibromatosis (DF). Recently, the Institut Gustave Roussy (IGR), Villejuif, France, reported a series of patients treated with a front-line conservative approach (no surgery and no radiotherapy). The disease remained stable in more than half of patients. This study was designed to evaluate this approach on the natural history of the disease in a larger series of patients.

Methods

A total of 142 patients presenting to the IGR or Istituto Nazionale Tumori (INT), Milan, Italy, were initially treated using a front-line deliberately conservative policy. Their progression-free survival (PFS) was observed and a multivariate analysis was performed for major clinical variables.

Results

Seventy-four patients presented with primary tumor, 68 with recurrence. Eighty-three patients received a “wait & see” policy (W&S), whereas 59 were initially offered medical therapy (MT), mainly hormonal therapy and chemotherapy. A family history of sporadic colorectal cancer was present in 8% of patients. The 5-year PFS was 49.9% for the W&S group and 58.6% for the medically treated patients (P = 0.3196). Similar results emerged for primary and recurrent DF. Multivariate analysis identified no clinical variables as independent predictors of PFS. In the event of progression, all patients were subsequently managed safely.

Conclusions

A conservative policy could be a safe approach to primary and recurrent DF, which could avoid unnecessary morbidity from surgery and/or radiation therapy. Half of patients had medium-term stable disease after W&S or MT. A multidisciplinary, stepwise approach should be prospectively tested in DF.

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Affiliations

  1. Department of Surgery, Istituto Nazionale Tumori, Milan, Italy

    Marco Fiore MD & Alessandro Gronchi MD

  2. Department of Surgery, Institut Gustave Roussy, Villejuif, France

    Françoise Rimareix MD & Sylvie Bonvalot MD, PhD

  3. Department of Biostatistics, Istituto Nazionale Tumori, Milan, Italy

    Luigi Mariani MD

  4. Department of Medical Oncology, Institut Gustave Roussy, Villejuif, France

    Julien Domont MD & Axel Le Cesne MD

  5. Department of Pathology, Istituto Nazionale Tumori, Milan, Italy

    Paola Collini MD

  6. Department of Radiotherapy, Institut Gustave Roussy, Villejuif, France

    Cecile Le Péchoux MD

  7. Department of Cancer Medicine, Istituto Nazionale Tumori, Milan, Italy

    Paolo G. Casali MD

Authors
  1. Marco Fiore MD
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  2. Françoise Rimareix MD
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  3. Luigi Mariani MD
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  4. Julien Domont MD
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  5. Paola Collini MD
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  6. Cecile Le Péchoux MD
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  7. Paolo G. Casali MD
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  8. Axel Le Cesne MD
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  9. Alessandro Gronchi MD
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  10. Sylvie Bonvalot MD, PhD
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Corresponding authors

Correspondence to Alessandro Gronchi MD or Sylvie Bonvalot MD, PhD.

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Fiore, M., Rimareix, F., Mariani, L. et al. Desmoid-Type Fibromatosis: A Front-Line Conservative Approach to Select Patients for Surgical Treatment. Ann Surg Oncol 16, 2587–2593 (2009). https://doi.org/10.1245/s10434-009-0586-2

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  • DOI: https://doi.org/10.1245/s10434-009-0586-2

Keywords

  • Medical Treatment
  • Imatinib Mesylate
  • Thoracic Wall
  • Isolate Limb Perfusion
  • Median Tumor Size